EBMT prospective observational study on allogeneic hematopoietic stem cell transplantation in T-prolymphocytic leukemia (T-PLL)

Preliminary data suggest that allogeneic stem cell transplantation (allo-SCT) may be effective in T-prolymphocytic leukemia (T-PLL). The purpose of the present observational study was to assess the outcome of allo-SCT in patients aged 65 years or younger with a centrally confirmed diagnosis of T-PLL. Patients were consecutively registered with the EBMT at the time of transplantation and followed by routine EBMT monitoring but with an extended dataset. Between 2007 and 2012, 37 evaluable patients (median age 56 years) were accrued. Pre-treatment contained alemtuzumab in 95% of patients. Sixty-two percent were in complete remission (CR) at the time of allo-SCT. Conditioning contained total body irradiation with 6 Gy or more (TBI6) in 30% of patients. With a median follow-up of 50 months, the 4-year non-relapse mortality, relapse incidence, progression-free (PFS) and overall survival were 32, 38, 30 and 42%, respectively. By univariate analysis, TBI6 in the conditioning was the only significant predictor for a low relapse risk, and an interval between diagnosis and allo-SCT of more than 12 months was associated with a lower NRM. This study confirms for the first time prospectively that allo-SCT can provide long-term disease control in a sizable albeit limited proportion of patients with T-PLL.Peer reviewe

Immunohistological localization of three basement membrane components in various forms of epidermolysis bullosa.

The skin biopsies of eight epidermolysis bullosa (EB) patients, representing epidermolytic, junctional and dermolytic forms of the disease were studied immunohistochemically using antibodies against collagen Types IV and V, and a proteoglycan. All these molecules are either basement membrane components or closely associated substances. In two types of EB simplex (subtype of the epidermolytic form) the splicing took place above the basement membrane, whereas the staining with all three antibodies remained localized to the floor of the blister. The herpetiform variant of EB simplex proved to be junctional, i.e. the separation occurred within the lamina lucida. One patient clinically classified as belonging to the junctional EB group, was found to have the epidermolytic form of the disease. In this case all antibodies were localized only on the floor of the blister. In the patients with the dermolytic form of EB, all the antibodies stained the roof of the blister. The immunofluorescence techniques are rapid and easy to perform and are therefore proposed as useful for routine clinical diagnosis

Generalized atrophic benign epidermolysis bullosa : either 180-kd bullous pemphigoid antigen or laminin-5 deficiency

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